QUALITY-OF-LIFE IN PATIENTS WITH CYSTIC-FIBROSIS

The majority of patients with cystic fibrosis (CF) will grow into adul thood. Despite this improvement in survival, the disease is progressiv e, especially with respect to the decline in pulmonary function, which in turn may have an impact on a patient's quality of life. In this st udy we evaluated the quality of life in CF adults and examined the rel ationship between quality of life and pulmonary function, exercise cap acity, and dyspnea. We assessed in 15 patients in stable clinical cond ition their forced expired volume in the first second (FEV(1)) inspira tory vital capacity (IVC), cycle exercise capacity (W-max), and subjec tive degrees of dyspnea during daily living (MRC dyspnea scale). Quali ty of life was assessed with the Sickness Impact Profile (SIP). A samp le of 100 healthy individuals, ranging in age from 18 to 30 years, ser ved as the control group. Mean (SD) age of the patients was 25.9 (7.3) years, FEV(1) was 38 (16) % predicted, IVC was 65 (17) % predicted, F EV(1) IVC ratio was 46 (10) %, and W-max was 90 (54) W. The overall SI P and physical SIP scores in CF patients were significantly higher tha n in the controls, indicating more impairment in overall and physical functioning in the patients than in the control group (P < 0.001). The psychosocial SIP score did not differ significantly between the two g roups. Overall SIP score correlated poorly with FEV(1) % predicted (r = -0.33; n.s) and IVC % predicted (r = -0.36; n.s.) but showed a bette r and significant relationship to the maximal exercise capacity (r = - 0.57; P < 0.05). MRC dyspnea scores showed a strong correlation with o verall SIP scores (r = 0.75; P < 0.001). These results show that CF af fects quality of life in adults primarily due to a limitation in physi cal functioning. Psychosocial functioning did not differ from that of healthy controls. Exercise capacity and dyspnea scores were related to the impairment in the quality of life. Therefore, the effects of prog rams aimed to improve exercise capacity and reduce dyspnea on CF patie nts' quality of life need to be evaluated. (C) 1997 Wiley-Liss, Inc.